title: Bosentan for idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is a debilitating, fatal, chronic fibrosing lung disease with no known effective therapy. Endothelin-1 may underlie the pathogenesis of lung fibrosis, therefore it was hypothesized that the oral dual endothelin receptor antagonist bosentan may have efficacy for the treatment of IPF. The BUILD-1 study evaluated the efficacy, safety and tolerability of bosentan in patients with IPF. Bosentan was associated with a trend toward delayed time to disease progression or death and improvement in quality-of-life, both of which were more pronounced in patients with a biopsy-confirmed IPF diagnosis. These observations are being investigated in the ongoing BUILD-3 trial.
authors.affiliation: University of California San Francisco, Department of Medicine, San Francisco, CA 94143-0120, USA. tking@medicine.ucsf.edu
authors.forename: Talmadge E
authors.initials: TE
authors.lastname: King
mesh_terms: D000818:Animals; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1 / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology; D011658:Pulmonary Fibrosis / Q000188:drug therapy* / Q000209:etiology; D016032:Randomized Controlled Trials as Topic; D013449:Sulfonamides / Q000627:therapeutic use*
chemical_list: D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1; D013449:Sulfonamides; D000077300:Bosentan